What does LMNA code for in and why is it so important in development of progeria?
LMNA codes for Lamina A-protein that acts as scaffolding on the inner side of the nucleus->farnesyl stays on lamina A->piles on=abnormal nuclear envelop
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15
Why does a person with a G6P deficiency need to eat corn starch?
corn starch is a glucose polymer that is broken down slowly = control glucose levels
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15
Summarize the two main roles of vWF in the process of clot formation
1.primary hemostasis- PLT adhesion; 2.secondary hemostasis- vWF complexes VIII->prolong half-life VIII (prevent its degradation)->complex IX->activate X
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15
Describe the process that leads to microangiopathic hemolytic anemia (schistocyte formation) in TTP
Discuss how Protein S and C work and their relevance in evaluating thrombophilia
Proteins S & C work together to prevent your blood from clotting too much; if low S&C = clot too much; P&C inactivate factors V/VIII = slow down clot formation
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trap
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star
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fairy
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lifesaver
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lifesaver
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rocket
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shark
Other team loses 5 points!
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baam
Lose 25 points!
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15
Contrast the pathogenesis of TTP with that of HUS
HUS: shiga toxin->ADAMTS 13 inhibition->failure to cleave vWF multimers; TTP: ADAMTS13 antibodies/congenital ADAMTS13 mutation->failure to cleave vWF multimers